膜性肾病
奥图穆马
贝里穆马布
医学
美罗华
免疫学
肾病综合征
伊库利珠单抗
表位
自身抗体
免疫原性
肾小球肾炎
抗原
抗体
补体系统
内科学
B细胞
肾
B细胞激活因子
作者
Gaetano La Manna,Olga Baraldi,Vania Cunia,Valeria Corradetti,Valeria Aiello,Marco Busutti,Lorenzo Gasperoni,Alessandra Spazzoli,Giorgia Comai
出处
期刊:PubMed
日期:2017-09-28
卷期号:34 (5): 113-118
摘要
The membranous nephropathy (MN) is the major cause of nephrotic syndrome in in the adult, account for 20% of cases with annual incidence is 1 in 100.000. In the past 10 years, the role of podocytes has been identified; environmental triggers in genetically predisposed patients can activate podocytes to exhibit antigenic epitopes (receptor of phospholipase A2, thrombospondin type 1) that become targets of specific autoantibodies with subsequent complement activation. The discovery of this mechanisms has opened new horizons in the therapy of MN and novel drugs are available with more specific mechanism of action. Rituximab, a monoclonal antibody directed against CD20 expressed by lymphocytes B, has been used in several trials and appears able to induce remission of nephrotic syndrome in 60% of patients (GEMRITUX trial) with similar risk profile. Nowadays it remains to define the most effective therapeutic pattern. In MN, the concept of targeting disease control, has permit novel therapies with specific blocking mechanisms (belimumab) and non-specific (ACTH) and new therapeutic options, such as ofatumumab, bortezomib and eculizumab, that have allowed to recognize pathological processes involved in the glomerular diseases.
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